Paget's Disease of Breast

Professional Reference articles are designed for health professionals to use. They are written by UK doctors and based on research evidence, UK and European Guidelines. You may find one of our health articles more useful.

Paget's disease of the breast was described by Sir James Paget in 1874. It is a rare type of cancer of the nipple-areola complex presenting as an eczematous lesion of the nipple and is often associated with an underlying in-situ or invasive carcinoma of the breast.[1]

Staging

Paget's disease of the nipple with no associated tumour is staged as carcinoma in situ. Otherwise, these are classified according to the size of the underlying tumour.

Management

The surgical treatment of Paget's disease is controversial. Mastectomy with or without axillary lymph node dissection has been regarded as the standard therapy for Paget's disease, even in the absence of other clinical signs of malignancy. High recurrence rates have been reported for patients treated with local excision alone, whose mammograms suggested in-situ changes confined to the nipple area. It has therefore been concluded that local excision alone is not an appropriate treatment for patients with Paget's disease of the nipple.[1]

However, with increasing diagnosis of early breast cancers, findings from multiple trials have shown that breast-conserving therapy is a feasible alternative for patients with disease limited to the central segment of the breast. Recent reports from several studies with long-term follow-up have shown that breast-conserving surgery is equivalent to mastectomy in terms of overall and disease-free survival in patients with breast cancer.[1, 9]

The addition of radiotherapy to breast-conserving surgery may be better in patients with Paget's disease of the breast associated with neither palpable mass nor abnormality on mammography (despite a high suspicion of underlying breast malignancy).[10] Medical management and chemotherapy for the underlying breast lesion may also be required.

Prognosis

In one study, 62% of patients with mammary Paget's disease presented with a detectable breast mass.

Even in patients with mammary Paget's disease and no underlying tumour, 30% may develop an invasive carcinoma at a later date and 20% of patients already have an associated in-situ carcinoma of the breast.[11]

Factors of unfavorable prognosis include the presence of a palpable breast tumour, lymph node enlargement, histological type of breast cancer, and age younger than 60 years.[1]

Approximately 25% of the cases are associated with an underlying in-situ or invasive neoplasm, particularly an adnexal apocrine carcinoma. Other associated malignancies include carcinomas of Bartholin's glands, urethra, bladder, vagina, cervix, endometrium and prostate.

It is a rare condition with only several hundred cases in the world reported. It most commonly appears in those aged 50-60 years and is more common in women.

It usually presents as chronic dermatitis of the groin, genitalia or perianal area, which is resistant to treatment. It is often associated with intense pruritus and long-standing lesions may cause pain and bleeding.

Related Information

Some background info, last june i started getting sever itching on my right nipple. ive had exzema my whole life so i thought it must be that. i treated it with my topical steroid cream but it didnt...

Article Information

The information on this page is written and peer reviewed by qualified clinicians.

Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical conditions. high-kick.ru has used all reasonable care in compiling the information but make no warranty as to its accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions. For details see our conditions.